Clinicopathologic, immunophenotypic, and molecular analysis of subcutaneous panniculitis‐like T‐cell lymphoma: A retrospective study in a tertiary care center

Abstract

Background Subcutaneous panniculitis‐like T‐cell lymphoma (SPTL) as strictly defined by World Health Organization‐European Organization for Research and Treatment of Cancer classification is a rare cytotoxic α/β T‐cell lymphoma, characterized by primary involvement of subcutaneous tissue mimicking panniculitis. Objectives To describe the clinicopathologic, immunophenotypic, and molecular features of SPTL. Methods A 10‐year retrospective study of 18 patients diagnosed with SPTL was thoroughly reviewed according to clinicopathology, immunophenotype, and T‐cell receptor (TCR) gene rearrangement. Results Of the 18 patients, 16 patients were definitely diagnosed with SPTL. The median age was 26 years (ranged 14‐53 years) with female predominance. Most patients presented with prolonged fever and subcutaneous nodules and/or plaques, usually located on lower extremities. 37.5% of patients had hemophagocytic syndrome. The main histopathology was lobular panniculitis with rimming of atypical lymphocytes highlighted by CD3+, CD8+, Beta‐F1+, granzyme B+, and Ki‐67 (50%‐90%). Monoclonal TCR gene rearrangement was found in 50% of patients and upper extremities involvement indicated a poor prognosis. Conclusion The correlation between clinicopathologic and immunophenotypic study is the most helpful method to give a precise diagnosis of SPTL. Rimming of CD8+ atypical lymphocytes highlighted by high Ki‐67 index is highly specific for the diagnosis of SPTL.

Primary Cutaneous Lymphomas in Thailand: A 10-Year Retrospective Study

“…An earlier study on mature T-cell and NK-cell lymphomas conducted in our hospital reported that the prevalence of SPTCL was higher than in other studies as well [ 28 ]. B-symptoms were observed in 86.2% of SPTCL patients in the present study, which was a similar rate to those reported in Thailand (87.5%) by Rutnin et al [ 29 ] and in Japan (81%) by Ohtsuka et al [ 30 ]. However, the prevalence of B-symptoms in our study was higher than in Europe (59%) [ 26 ].…”

“…We observed that HPS developed in 13.8% of SPTCL cases. Studies by Willemze et al [ 26 ], Ohtsuka et al [ 30 ], Rutnin et al [ 29 ], and Lee et al [ 31 ] exhibited wide variations of the HPS associated with SPTCL in Europe (17%), Japan (45%), Thailand (37.5%), and Korea (14%), respectively. In addition, laboratory abnormalities, including elevated LDH, elevated liver enzymes, anemia, and leukopenia, were observed, similar to in previous reports [ 26 , 29 , 31 , 32 ].…”

Chidamide induces long-term remission in rare subcutaneous panniculitis-like T-cell lymphoma: An unusual case report and literature review

“…Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a distinct subset of primary cutaneous T-cell lymphoma that preferentially infiltrates subcutaneous adipose tissue, mimicking panniculitis, accounting for approximately 1%-2.3% of cutaneous lymphoma. 1 The typical manifestations of SPTCL are subcutaneous nodules and/or plaques of the extremities and/or trunk, 2,3 but it can also involve extracutaneous viscerals, such as bone marrow, 4 breast, 5 liver, 5 spleen, 5 mesenteric fat, 6 lymph node, 7 lung, 8,9 and muscle. 10 We report a 47-year-old woman with SPTCL who presented with serious pulmonary diseases as the first symptom, gradually she appeared swelling of right thigh SPTCL, although various treatments such as chemotherapy, radiotherapy, immunosuppressive agents, and hematopoietic stem cell transplantation (HSCT) have been reported.…”

Therapy of the Resistant Forms of Subcutaneous Panniculitis-Like T-Cell Lymphoma

“…Частота встречаемости заболевания составляет не более 1 % от всех неходжкинских лимфом: за 10 лет наблюдения крупными группами по изучению и лечению кожных лимфом описано не более 18 случаев [6,8]. ППТКЛ встречается, как правило, среди больных молодого возраста (медиана возраста -36 лет), примерно в 20 % случаев -20 лет и моложе, чаще среди женщин (соотношение мужчины : женщины = 2,2 : 1) [7,9]. Этиология заболевания в настоящее время не установлена, известны случаи развития ППТКЛ после органной трансплантации, иммуносупрессивной терапии [10, | КЛИНИЧЕСКИЕ НАБЛЮДЕНИЯ | CASE REPORTS | 11].…”

“…Опухолевые клетки имеют α/β-цитотоксический иммунофенотип: экспрессируют βF1 + , CD3 + , CD8 + , TIA1 + , perforin+, granzyme B + , отсутствует экспрессия CD4 -, CD56 -, CD30 -, может наблюдаться потеря пан-Т-клеточных антигенов CD2, CD5 и CD7, пролиферативная активность (Ki-67) варьирует от 10 до 90 % [16]. Молекулярно-генетические исследования определяют клональную реаранжировку генов β-цепи Т-клеточного рецептора в 100 % случаев и генов γ-цепи Т-клеточного рецептора -в 50-80 % [7,13,17].…”